Prevention and Management of Periampullary Tumors in Familial Adenomatous Polyposis - Open Access Library Journal

OALibJ > Vol.3 No.1, January 2016

Prevention and Management of Periampullary Tumors in Familial Adenomatous Polyposis ()

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DOI: 10.4236/oalib.1102338 777 Downloads 1,515 Views

Author(s)

Naoki Hashimoto

Affiliation(s)

School of Medicine, Kinki University, Osaka Sayama, Japan.

ABSTRACT

Familial adenomatosis polyposis (FAP) is one of two well described forms of hereditary colorectal cancer. The primary cause of death from this symdrome is colorectal caner which develops usually by the fifth decade of life. The overall survival of FAP was significantly improved by screening by genetic testing and endoscopy with prophylactic surgery. Almost all patients with FAP develop duodenal polyposis most of which occurs in periampullary. The second leading cause of death in FAP is duodenal adenocarcinoma. Between 5% and 10% of FAP patients die from duodenal cancer, usually periampullary in origin. Therefore, I will discuss the clinicopathological features, management and prevention of duodenal neoplasma in patients with FAP.

KEYWORDS

Familial Adenomatous Polyposis (FAP), Duodenal Adenocarcinoma, Spigelman Classfication, Pancreas-Preserving Total Duodenectomy (PPTD)

Share and Cite:

Hashimoto, N. (2016) Prevention and Management of Periampullary Tumors in Familial Adenomatous Polyposis. Open Access Library Journal, 3, 1-5. doi: 10.4236/oalib.1102338.

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